More severe SLE found in Japanese patients who also have PAH: Study

Initial treatment to manage SLE, and vasodilators for advanced disease

Patricia Inacio, PhD avatar

by Patricia Inacio, PhD |

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Among people with systemic lupus erythematosus (SLE), those who develop pulmonary arterial hypertension (PAH), or high blood pressure in the vessels that supply the lungs, appear to be younger at diagnosis and have more severe disease, a study in Japan has found.

In SLE patients with PAH, immune-suppressing glucocorticoids are the treatment of choice, followed by vasodilators, or therapies that widen blood vessels to lower blood pressure. In about one in five patients, these drugs are taken in combination.

“The initial treatment goal among SLE-PAH patients appears to be mitigation of SLE, which is in line with the Japanese guidelines for the treatment of PAH, whereas vasodilators are preferred more in the subsequent lines of therapies,” researchers wrote.

The study, “Prevalence, patient characteristics and treatment patterns among systemic lupus erythematosus–pulmonary arterial hypertension patients in real-world clinical practice: A retrospective analysis of Medical Data Vision Database in Japan,” was published in the journal Modern Rheumatology.

PAH is a cardiovascular condition in which the blood vessels that supply the lungs become narrower than normal, leading to an increase in blood pressure that makes the heart work harder to pump out blood.

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PAH most prevalent as complication of SLE in Asia

The condition is often associated with the presence of other diseases. While the disease that is most commonly associated with PAH varies worldwide, PAH seems to occur most commonly as a complication of SLE in Asian regions.

As PAH is one of the most common causes of death and illness in SLE patients, a timely diagnosis and treatment is key. However, real-world evidence on the prevalence, patient characteristics, and treatment strategies for PAH-SLE patients is limited.

To known more, a team led by researchers at the National University of Singapore and Janssen Pharmaceutical Asia Pacific, in Japan, conducted a retrospective analysis of data collected from more than 29,000 SLE patients from 2018 to 2020. The data came from Japan’s Medical Data Vision, the country’s largest hospital-based claims database with more than 40 million patients.

Among the 29,077 patients included in the analysis, 114 developed PAH after their lupus diagnosis, indicating a prevalence of 0.392%. Nearly half of SLE-PAH patients (49%) developed PAH within one year of their SLE diagnosis.

Compared with patients with SLE alone, those who also had PAH were more likely to be women (88% vs. 72%), experience longer follow-ups (mean 3.8 vs. 2.2 years), and to have severe lupus (61% vs. 25%).

The first line of treatment for PAH-SLE patients was predominantly glucocorticoids (58%), which are used to dampen the excessive immune response that drives lupus, but the use of these medications decreased for later lines of therapy.

In turn, vasodilators, which are standard to treat PAH, were less frequently used as a first treatment (27%), but were used more commonly as second-line (38%) and third-line therapies (44%). Generally, patients who transitioned from first-line glucocorticoids to second-line vasodilators did so after a median of 1.6 months.

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Most patients on mix of combination therapies

In general, most patients received a mix of combination therapies, including glucocorticoids and immunosuppressants; vasodilators, glucocorticoids, and immunomodulators; or vasodilators and glucocorticoids.

The most common vasodilators used as first- and second-line therapies belonged to the endothelin receptor antagonists and nitric oxide class of treatments, which were collectively prescribed to more than 70% of patients who received vasodilators. Prostacyclins were the commonly used third-line therapies.

“In Japan’s real-world practice, the initial treatment goal is SLE management, while vasodilators are preferred in advanced diseases,” the researchers wrote.

However, “given that only a small number of patients with SLE–PAH were identified in this study, future research with a large sample size is recommended,” they added.